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Mitral Valve Prolapse (MVP)

BASICS

Description

  • Billowing of one or both mitral valve leaflets into the LA during ventricular systole
  • Morphology: Classic vs Nonclassic MVP
  • Often asymptomatic, but can cause palpitations, MR, or stroke

Synonyms: systolic click-murmur syndrome, floppy valve syndrome, Barlow syndrome

EPIDEMIOLOGY

  • Prevalence: 1–3%
  • Equal gender distribution
  • Typically seen in adults

ETIOLOGY & PATHOPHYSIOLOGY

  • Myxomatous degeneration: thickened spongiosa, abnormal collagen, chordae elongation
  • Primary MVP: sporadic or familial
  • Secondary MVP: associated with:
  • Connective tissue disorders: Marfan, Ehlers-Danlos, Loeys-Dietz
  • Congenital heart disease: ASD, Ebstein anomaly
  • Papillary/chordae dysfunction: infarction, trauma, endocarditis, HCM

Genetics

  • Autosomal dominant (MMVP1, MMVP2, MMVP3)
  • X-linked (filamin A gene, Xq28)

RISK FACTORS

  • Heritable and congenital heart conditions
  • Lean BMI

ASSOCIATED CONDITIONS

  • MR, stroke, endocarditis
  • May also be seen with von Willebrand disease, hypomastia, skeletal abnormalities

DIAGNOSIS

History

  • Most patients asymptomatic
  • Symptoms:
  • MVP: palpitations, chest pain, orthostasis, fatigue, panic
  • MR: dyspnea, orthopnea, PND

Physical Exam

  • Midsystolic click, Β± late systolic murmur at apex
  • Murmur shifts with:
  • ↓ preload: click/murmur β†’ earlier (Valsalva, standing)

  • ↑ preload: click/murmur β†’ later (squatting)

  • MR murmur: holosystolic, radiates to axilla, S3 = severe MR

Differential Diagnosis

  • Ejection click, MR, TR, HCM, papillary dysfunction

Diagnostic Tests

  • TTE: diagnostic modality of choice
  • TEE (3D): for surgery planning or poor TTE windows
  • ECG: often normal; Β± ST-T changes, QT prolongation
  • Ambulatory monitoring: if palpitations present
  • MRI/EP study: if high SCD risk

Interpretation

  • MVP = β‰₯2 mm leaflet displacement into LA during systole
  • Classic MVP: thickening >5 mm
  • Nonclassic MVP: thickening <5 mm
  • Flail leaflet: extreme form, often with torn chordae

TREATMENT

General Measures

  • Reassurance for mild, asymptomatic MVP
  • Avoid caffeine, alcohol, nicotine
  • Orthostasis: salt/fluid intake, compression stockings

Medications

  • Aspirin 75–325 mg:
  • For TIAs or high-risk MVP (thickening >5 mm)
  • Warfarin:
  • MVP + stroke/TIA + MR, AF, thrombus, or valve redundancy
  • Ξ²-blockers: for palpitations or arrhythmia

REFERRAL

  • Cardiology: if symptoms or high-risk features
  • EP: significant arrhythmia
  • CT surgery: severe MR or surgical candidacy
  • Genetics: if syndromic/connective tissue disorder suspected

SURGERY/PROCEDURES

  • MVP + myxomatous degeneration β†’ most common cause of primary MR needing surgery
  • Surgery Indicated for:
  • Severe MR + symptoms
  • Asymptomatic: EF ≀60%, LVESD β‰₯40mm

  • AF or pulmonary HTN in severe MR

  • MV repair > replacement (better outcomes)

ONGOING CARE

Follow-Up

  • MVP with no MR: TTE every 3–5 yrs
  • MVP + MR or symptoms: Yearly eval + TTE

Patient Education

  • MVP usually benign
  • No pregnancy restrictions
  • Report new symptoms
  • Avoid sports if:
  • Moderate LV enlargement, arrhythmia, prolonged QT
  • Syncope, prior SCD, aortic root dilation

PROGNOSIS

  • Excellent for asymptomatic MVP
  • SCD risk: rare but elevated (0.14 per 100 pt-years)

COMPLICATIONS

  • MR, HF, pulmonary HTN
  • AF, arrhythmias: PACs, SVT, VT, SCD
  • TIA/stroke, infective endocarditis

ICD-10

  • I34.1: Nonrheumatic mitral valve prolapse
  • I05.8: Other rheumatic mitral valve diseases

Clinical Pearls

  • MVP = leaflet billowing into LA during systole
  • Click Β± late systolic murmur is hallmark auscultation
  • Echocardiogram required for diagnosis
  • Etiology is often multifactorial: myxomatous, syndromic, post-MI, etc.